ADAMTS-13 deficiency following Hemiscorpius lepturus scorpion sting

Hemiscorpius lepturus is a lethal scorpion with potentially cytotoxic venom. Various degrees of local and systemic toxicity have been observed after its envenomation ranging from local erythema to disseminated intravascular coagulation,renal failure and severe pulmonary hemorrhage. In this case report,we report on a seven-year-old patient who developed the hemolytic uremic syndrome (HUS) after being stung by the scorpion H. lepturus. This condition is characterized by microangiopathic hemolytic anemia,thrombocytopenia,increased serum levels of lactate dehydrogenase and uremia. We evaluated the causes of HUS and found that the levels of C3,C4,CH50 and H factors were normal,but the activity of Von Willebrand factor cleaving protease was decreased (less than 5% of the normal activity). The patient improved after administering therapy with plasma exchange.